Congenital Heart Disease

1
Congenital Heart Disease
Greg Gordon MD
2 Feb 05 24 May 06 31 May 07
2
Training for Career in Pediatric Cardiac
Anesthesia
Specific Fellowship Rare
Suggested training (US UK)

• Pediatric Anesthesia 12 months
• Adult Cardiac Anesthesia 6 months
• Pediatric Cardiac Anesthesia 6 months
• Pediatric Critical Care 6 months

Baum V De Souza DG. Pediatric Anesthesia
17407, 2007 White MC Murphy TWG. Pediatric
Anesthesia 17421, 2007
3
?

• PDA ligations
• Murmurs preop
• CHD patients for
• noncardiac surgery

4
Adults with CHD in US today
2,140,000
Growing 5 per year
Cahalan MK. Anesthetic Management of Patients
with Heart Disease. IARS 2003 Review Course
Lectures
5
3 y/o with TOF
s/p right BTS
For dental restorations

• Turns blue with crying
• Scheduled to undergo cardiac repair
• in 3 months
• SpO2 93
• Systolic ejection murmur
• Slight clubbing of fingers
• Hct 52

Tammy
6
(Recent oral board case)
5 y/o for TA
Systolic murmur

• VSD
• Needs surgical closure
• Cardiologist recommended TA first

Victor
7
11 y/o with tricuspid atresia
s/p Fontan procedure
For scoliosis repair

• Temporary BTS at age 3 weeks
• Modified Fontan at age 3 years
• Meds digoxin, captopril
• SpO2 88 on RA, 98 in O2
• P 67, BP 99/42
• First degree AV block

Fran
8
Objectives Participants will be able to more
intelligently discuss

• Newborn heart and lungs
• Initial evaluation the childs heart
• Pathophysiology of selected CHDs
• Anesthetic implications of CHD

9
The Newborn Heart
CHOP Duct Busters
Provide service to 17 area NICUs Send team of 2
each surgeons anesthesia providers (attendin
g CRNA) nurses Operate within 24 - 48
hours Monday Friday No weekends Reimbursemen
t exceeds other cardiac services
Susan Nicholson and Gould DS et al Pediatrics
2003 1121298-1301
10
The Newborn Heart
Foramen Ovale
Functional closure first hours as LAP gt
RAP Probe-patent 50 of 5-year-olds 25 of
20-year-olds Paradoxical embolus
11
The Newborn Heart
Ventricular tissue

• Fewer myocytes
• Greater proportion of connective tissue
• Relative RVH

So

• Decreased compliance
• More sensitive to preload

12
The Newborn Heart

• Near peak of Starling curve
• Stroke volume relatively fixed
• C.O. relatively heart rate dependent

Normally near peak of Starling curve Stroke
volume relatively fixed C.O. relatively heart
rate dependent
13
The Newborn Heart
Ca
Newborn myocardium derives relatively high
fraction of activator Ca from the extracellular
pool, so
Beware Ca channel blockers
14
The Preterm Infant Heart
More sensitive to depressant effects of inhaled
agents Decreased response to catecholamines
Relatively high PVR persists
Pulmonary vasculature more sensitive to
vasoconstriction by
Hypoxia Acidosis Hypercarbia
15
CHD Pearl
murmur in newborn benign disease
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Initial evaluation of childs heart
History To determine

• Level of function
• CHF

17
Initial evaluation of childs heart
History - cyanosis

• Turn blue?
• At rest?
• When crying?
• Passes out?
• Stops playing and squats

18
Initial evaluation of childs heart
History - CHF
Run around like crazy? Like sibs? Or tends to be
quiet, slow? Infant feeding behavior Slow to
finish bottle? Sweats when nursing? Eyes puffy in
the morning?
19
Initial evaluation of childs heart
Physical exam

• Listen to heart first when/if infant quiet
• (warm stethoscope)
• First concentrate on S1 and especially S2
• Louder than normal?
• Split normally?
• Systolic murmur
• Starts after or obscures S1?
• Diastolic murmur?
• Widely radiating murmur?
• Palpate liver
• BP in arm and leg
• Tongue - cyanosis

20
CHD Pearl
Sudden CHF in healthy 10-day-old complicated
coarct
21
General Approach to CHD Patient

• Define cardiovascular pathology
• Predict pathophysiology
• Determine hemodynamic goals
• Anticipate emergency treatments

Cahalan MK. Anesthetic Management of Patients
with Heart Disease. IARS 2003 Review Course
Lectures
22
Dont worry
23
Almost any anesthetic technic may be used in any
CHD patient
if

• the anesthesiologist understands
• the pathophysiology of the lesion and
• the pharmacology of the drugs employed.

24
Normal Neonate 1 week
SVC
PV
60
99
LA
RA
m2
m4
65
RV
LV
30/3
80/5
65
99
MPA
Ao
65
99
30/12 m18
80/50
25
Some basic definitions
physiologic L to R shunt
lungs to lungs shunt
Blood that is returning to the heart from the
lungs is recirculated back to the lungs without
going out to the rest of the body.
26
Some basic definitions
physiologic R to L shunt
body to body shunt
Blood that is returning to the heart from the
body is recirculated directly back to the body
without going to the lungs to be oxygenated.
27
Some basic definitions
effective pulmonary blood flow
body to lungs flow
Blood that is returning to the heart from the
body that is actually directed to the lungs to
be oxygenated.
28
Some basic definitions
Nonrestrictive VSD
VSD large enough that pressure equalizes in the
two ventricles (no pressure gradient can be
maintained) LV pressure RV pressure
29
Premature
1 week old
PV
SVC
28 weeks EGA
RA
LA
96
65
RV
LV
65/10
65/12
65
96
Ao
MPA
PDA
65/25
65/30
80
92
30
to R arm head
To L arm
MHMC PDA ligation
31
CHD Pearl
blue newborn no airway or breathing problem
quiet heart decreased PBF lesion (TOF)
32
Tetralogy Of Fallot
Most common cyanotic lesion NB cyanosis plus
quiet heart Diminished pulmonary blood flow Ao
ejection click Hypercyanotic tet spells
tachypnea, pallor, LOC, less murmur
Tammy
33
(No Transcript)
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(No Transcript)
35
3 y/o with TOF
s/p right BTS

• Define cardiovascular pathology
• Predict pathophysiology
• Determine hemodynamic goals
• Anticipate emergency treatments

Tammy
36
Tetralogy Of Fallot

• Essentially a duality
• severe RVOT obstruction plus
• nonrestrictive VSD

• With anatomic consequences
• RVH
• Overriding aorta

Tammy

• And physiologic consequences
• R to L shunt
• Diminished pulmonary blood flow

37
(No Transcript)
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Tetralogy of Fallot
SVC
40
96
RA
LA
m5
m4
RV
LV
85/6
85/5
40
85
MPA
50
Ao
40
15/10
85/45
39
Tetralogy Of Fallot
s/p right BTS?
Blalock-Taussig Shunt
Tammy
40
Thomas-Blalock-Taussig Shunt
Vivien Thomas
Alfred Blalock
Helen Taussig
Vivien Thomas, Partners of the Heart, 1998
and Something the Lord Made - Best Made-for-TV
Movie, 2004
41
November 29, 1944 Thomas-Blalock-Tuassig
42
Dr. Blalock does the Blalock (Johns Hopkins)
43
Systemic to Pulmonary Shunts
44
Tetralogy Of Fallot
Maintain adequate tissue oxygenation

• Avoid increasing O2 demand
• Maintain SVR, systemic BP
• Minimize PVR

Avoid dehydration, especially if polycythemic
Tammy
Oral premed/induction midazolam ketamine
45
Free written board answer
Speed of induction

• R-gtL shunt
• Inhalational slower
• IV faster

• L-gtR shunt
• Inhalational maybe faster
• IV slower

But probably not clinically important
Tanner et al. Anesth Analg 64101, 1985
46
Beware blunted chemoreceptor response to
hypoxemia
Tammy
47
Beware
VDVT may be 0.6

• And increase with
• start of mechanical ventilation
• too much PEEP
• hypovolemia

Tammy
ETCO2 ltlt PaCO2
48
Tetralogy Of Fallot
Minimize R-gtL Shunt
MAINTAIN SVR

• ketamine
• phenylephrine

Tammy
49
Tetralogy Of Fallot
Minimize RVOT obst PVR

• oxygen
• beta blocker ready

• Maybe
• nitroglycerin
• phentolamine
• tolazoline
• prostaglandin E1
• nitric oxide

Tammy
50
Tetralogy Of Fallot
And of course

• No Air in lines

Maybe no N2O
and
infectious endocarditis prophylaxis
Tammy
51
Infectious Endocarditis Prophylaxis
Negligible Risk
Ostium secundum ASD 6 months after uncomplicated
repair of ASD VSD PDA MV prolapse without
regurge or thick leaflets Normal murmur (need
ECHO in adult) Pacemakers and ICDs Hx of
CABG Kawasaki or RF without valve problem
Dajani A, Taubert K, et al. Prevention of
bacterial endocarditis. Recommendations by the
American Heart Association. JAMA 2771794-1801,
1997
52
Infectious Endocarditis Prophylaxis
High Risk

• Prosthetic heart valves
• Hx bacterial endocarditis
• Complex cyanotic CHD
• Surgical systemic pulmonary shunts

JAMA 2771794-1801, 1997
53
Infectious Endocarditis Prophylaxis
Moderate Risk
Other CHD PDA VSD Ostium primum ASD bicuspid
Ao valve coarctation Acquired valve disease MVP
with regurge or abnl leaflets Hypertrophic
cardiomyopathy
JAMA 2771794-1801, 1997
54
Infectious Endocarditis Prophylaxis
Prophylaxis Recommended
Dental procedures with bleeding Respiratory
mucosa surgery T A rigid bronchoscopy GU
surgery prostate urethral dilation cystoscopy
JAMA 2771794-1801, 1997
55
Infectious Endocarditis Prophylaxis
Prophylaxis Recommended for High Risk Patients
GI surgery esophageal sclerotherapy,
dilation ERCP with obstruction intestinal
mucosa Optional flexible bronchoscopy vaginal
delivery
JAMA 2771794-1801, 1997
56
Infectious Endocarditis Prophylaxis
NOT Recommended
Dental procedures unlikely to cause
bleeding Endotracheal intubation Typanostomy tube
insertion Cesarean section DC TL
JAMA 2771794-1801, 1997
57
Tetralogy Of Fallot
mainly
maintain
SVR
Tammy
58
Tetralogy Of Fallot
Treatment of Tet Spell

• Knee-chest position
• O2
• Morphine 0.1-0.2 mg/kg IM,IV
• Phenylephrine gtts increase systolic BP 20-40
  mmHg
• Beta blockade, e.g. propanolol titrate to 0.1
  mg/kg
• ABG NaHCO3 if necessary
• Surgery

59
CHD Pearl
blue newborn no airway or breathing problem
hyperactive heart TGA
60
(Recent oral board case)
5 y/o for TA
Systolic murmur

• VSD
• Needs surgical closure
• Cardiologist recommended TA first

Victor
61
(No Transcript)
62
Newborn VSD
Most common lesion 2/3rds close
spontaneously Small VSD Definite murmur Will
probably close Large VSD No murmur No
problems Home with Mom
CHF symptoms by 4-8 weeks
63
VSD
nonrestrictive
SVC
98
60
LA
96
RA
m6
m12
80
RV
LV
90/8
90/10
94
88
94
Ao
MPA
90/60
90/35
64
(No Transcript)
65
Nonrestrictive VSD
L-gtR shunt
Pulmonary to System Flow Ratio
Victor
SaO2 SvO2
__________
QPQS
SpvO2 SpaO2
94 - 60
_______

98 - 88

3.41
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Nonrestrictive VSD
Besides, of course

• No Air in lines

Maybe no N2O
and
Victor
infectious endocarditis prophylaxis
69
Proper management of the physiologic
abnormalities is more important than the choice
of specific anesthetic and pharmacologic
approaches.
70
Nonrestrictive VSD
Maintain PVR
Normal ventilation (paCO2 40s)
FIO2 lt 1
Victor
Lower SVR better
Major inhalational agents
Thiopental, propofol
71
11 y/o with tricuspid atresia
s/p Fontan procedure
For scoliosis repair

• Temporary BTS at age 3 weeks
• Modified Fontan at age 3 years
• Meds digoxin, captopril
• SpO2 88 on RA, 98 in O2
• P 67, BP 99/42
• First degree AV block

Fran
72
Tricuspid Atresia
3rd most common cyanotic CHD 1. TOF 2. TGA

• Type IB most common
• Small VSD (and RV)
• PS

Fran

• 20 extracardiac abnormalities
• GI
• Musculoskeletal

• Cyanosis
• Mixing in LA
• Decreased PBF
• Spells

73
(No Transcript)
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Modified Bidirectional
75
Modified
76
Age 5 years
16/10
16/12
88/6
77
11 y/o with tricuspid atresia s/p Fontan procedure
Potential problems during scoliosis repair

• Hypoxemia
• Hypovolemia
• Low PBF

• CHF
• Volume shifts
• Anemia
• Hypertension

Fran
Paradoxical embolus
Thrombosis Vena cavae RA Pulmonary
arteries
78
11 y/o with tricuspid atresia s/p Fontan procedure
Goals during scoliosis repair

• Monitor RA pressure
• RA catheter
• Maintain starting pressure

Maintain systemic BP near baseline
Fran
Minimize myocardial depressants
NO AIR IN LINES No N2O
Relatively high FIO2
Normal Hct
79
Age 5 years
16/10
16/12
88/6
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For more cool stuff about CHD check out the
lesson and fun Quiz at
http//metrohealthanesthesia.com/edu/ped/chd1.htm
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Now we can more intelligently discuss

• Newborn heart and lungs
• Initial evaluation the childs heart
• Pathophysiology of selected CHD
• Anesthetic implications of CHD